AB1472 THE PECULIARITIES OF THE CLINICAL MANIFESTATIONS OF FAMILIAL MEDITERRANEAN FEVER ACCOMPANIED WITH LUPUS-LIKE SYNDROME
Authors: M. Efraimidou, M. Osipyan, L. Sevoyan, S. Bazeyan, V. Vardanyan, K. Ginosyan
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> The most common autoinflammatory disease which occurs in Armenian ethnos is familial Mediterranean fever (FMF). FMF is a hereditary disorder characterized by recurrent attacks of fever and serosal inflammation. The main clinical manifestations of FMF are recurrent fever, abdominal, chest and joint pain, erysipelas-like skin lesion but FMF also has a lot of other rare and systemic clinical manifestations. Sometimes it is difficult to distinguish if the patient has only FMF or the overlap of FMF and other rheumatological diseases: systemic lupus erythematosus (SLE), systemic vasculitis, spondyloarthropathy, etc. <h3>Objectives</h3> The purpose of the study is to reveal non-classic clinical manifestations of FMF, analyze and compare the course of FMF in these cases. <h3>Methods</h3> 100 Armenian patients with genetically confirmed FMF, who also fulfilled Tel-Hashomer classification criteria were included in this study. Molecular-genetic detection of 26 MEFV mutations which are more common among Armenians was carried out. Only 13 patients from 100 had systemic clinical manifestations like lupus, so we continued investigation and determine ANA, anti-dsDNA antibodies, which were positive. All patients with lupus-like syndrome fulfilled diagnostic criteria of SLE by ACR. Comparison of peculiarity of course of FMF was done between 13 patients - with both lupus-like syndrome and FMF (group I) and 13 patients only with FMF (group II). <h3>Results</h3> There were 11 female and 2 male patients in the first group. The mean age was 40,06±2,46. The 15.4% of the patients were affected with FMF and lupus-like syndrome/SLE parallelly and in 30.8% cases the onset of SLE was prior to the clinical presentation of FMF. They all had systemic clinical manifestations such as erythema (92.3%), livedo reticularis (69.2%), photosensitivity (69.2%), Raynaud's phenomenon (23.07%). Though the frequency of FMF attacks in the I group was more than in the II group, the high temperature 39-40 C° was viewed in 23% of the I group and in 46% of the II group and the duration of attack days was respectively 1-3 and 1-5. We noticed that thoracalgia and monoarthritis were viewed less frequently in the I group than in the II, 7.7% and 15.4% respectively. The difference between the clinical manifestations is presented below (Table 1). The prevalent mutation of MEFV gene in both groups was M694V- in 19 patients (73%), from which 3 (11,5%) were homozygote and 23 (88,5%) were heterozygote. <h3>Conclusion</h3> In conclusion, both diseases FMF and SLE have common clinical manifestations: fever, arthralgia, arthritis, skin involvement, pleuritis, pericarditis. The course of FMF with coexisting SLE is milder: these patients have lower temperature, shorter duration of attack days; thoracalgia and monoarthritis also appear less frequently. Further investigations are needed to reveal the peculiarity of duration of FMF with co-exciting of systemic rheumatic diseases. <h3>References</h3> [1]B. Schreiber, H. Lachmann, C. Mackworth-Young. Possible familial Mediterranean fever in a Caucasian patient with systemic lupus erythematosus. Lupus, 2008;17(8):752-3. doi: 10.1177/0961203308089449. [2]T. Klein, Sh. Tiosano, Y. Gilboa, Y. Shoenfeld, A. Cohen, H. Amital. The coexistence of familial Mediterranean fever (FMF) in systemic lupus erythematosus (SLE) patients - A cross sectional study. Lupus, 2021;30(7):1094-1099. doi: 10.1177/09612033211004726. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.